RHEUMATIC VALVULAR DISEASE

OBJECTIVES:

Definition of rheumatic heart disease.

Pathogenesis of RHD.
Clinical features of RHD.
Criteria for diagnosis of RHD.
Rationale:
DEFINITIONS:-

Stenosis:
Is the failure of a valve to open completely,
obstructing forward flow.
Almost always a chronic process caused
by a primary cuspal abnormality (e.g.,
calcification or valve scarring).
Insufficiency (regurgitation):
Failure of a valve to close completely,
thereby allowing reversed flow.
Result from either intrinsic disease of the
valve cusps or distortion of the supporting
structures.
RHEUMATIC VALVULAR DISEASE:

Rheumatic fever (RF):

Is an acute, immunologically mediated,
multisystem inflammatory disease that
occurs a few weeks after an episode of
group A β -hemolytic streptococcal
pharyngitis; it can also rarely occur with
streptococcal infections at other sites (e.g:
skin).
PATHOGENESIS:

Acute RF is a hypersensitivity reaction

induced by host antibodies elicited by
group A streptococci.
M proteins of certain streptococcal
strains induce host antibodies that
cross-react with glycoprotein antigens
in the heart, joints, and other tissues.
ACUTE RHEUMATIC FEVER
causes changes in the endocardium, myocardium, and
epicardium.
chronic rheumatic heart disease
is almost always caused by deformity of the heart
valves, particularly the mitral and aortic valves.
Acute rheumatic heart disease (RHD):
Is the cardiac manifestation of RF and is
associated with inflammation of the valves,
myocardium, or pericardium.
During acute RF, discrete inflammatory
lesions are found in various tissues
throughout the body.
Within the heart these are called Aschoff
bodies and are pathognomonic for RF.
Aschoff bodies can be found in any of the
three layers of the heart-pericardium,
myocardium, or endocardium -so- called
pancarditis.
Chronic RHD:
Characterized by organization of the acute
inflammation and subsequent scarring.
The cardinal anatomic changes of the
mitral valve include leaflet thickening,
commissural fusion and shortening, and
thickening and fusion of the chordae
tendineae.
The functional consequence of RHD is
valvular stenosis and regurgitation
(stenosis tends to predominate).
The mitral valve alone is involved in 70%
of cases of RHD, with combined mitral and
aortic disease in another 25%; the
tricuspid valve is usually less frequently
and less severely involved, and the
pulmonic valve almost always escapes
injury.
With tight mitral stenosis, the left atrium
progressively dilates owing to pressure
overload , causing thrombosis, and
formation of large mural thrombi is
common.
Long-standing passive venous congestion
gives rise to pulmonary vascular and
parenchymal changes typical of left-sided
heart failure.
In time, this leads to right ventricular
hypertrophy and failure. With pure mitral
stenosis, the left ventricle generally is
normal
CLINICAL FEATURES:

Acute RF :
1.Appears most often in children aged 5 to
15 years
80% in Children
20% occur in adults : arthritis
Symptoms occur two to three weeks after
an episode of streptococcal pharyngitis.
2.The predominant clinical manifestations
are arthritis and carditis.
Arthritis are migratory poly arthritis
accompanied by fever and it subsides
spontaneously, leaving no residual
disability.
3.Clinical features of the carditis include
pericardial friction rubs and arrhythmias.
4.Myocarditis can be so severe that
resulting cardiac dilation causes functional
mitral insufficiency and even CHF.
Although cultures are negative for
streptococci at the time of symptom onset,
serum titers of antibodies against one or
more streptococcal antigens(e.g.,
streptolysin O or DNAase) usually are
elevated
After an initial attack and the generation of
immunologic memory, patients are
increasingly vulnerable to disease
reactivation with any subsequent
streptococcal infections.
Chronic RHD :
1.Usually does not cause clinical
manifestations for years after the initial
episode of RF.
2.The signs and symptoms of valvular
disease depend on which valve(s) are
involved.
3.The mitral valve is the one most
commonly involved and its stenosis is the
most common manifestation.
4.Patients with chronic RHD often have
arrhythmias (particularly atrial fibrillation in
the setting of mitral stenosis)
5.Thromboembolic complications, and an
increased risk of subsequent infective
endocarditis.
DIAGNOSIS OF ACUTE RHD IS MADE
BY:
Serologic evidence of a previous streptococcal infection, in
conjunction with two or more of the following Jones criteria:

(1) Carditis.
(2) Migratory polyarthritis of the large joints
(3) Subcutaneous nodules(The nodules appear over the
extensor surfaces of (elbows, knees & ankles ;non-tender &
not attached to the overlying skin).
(4) Erythema marginatum of the skin.( red to pink, non-
pruritic macules or papules on the trunk & proximal limbs,
but never on the face.Spreads outwards with raised
erythematous edges & central clearing).
(5) Sydenham chorea, a neurologic disorder with involuntary
purposeless, rapid movements.
 Minor criteria such as:-
fever, arthralgias, ECG changes, or
elevated acute phase reactants also can
help support the diagnosis.
WHAT ARE THE CRITERIA FOR
DIAGNOSIS?
We have major criteria which are:
Evidence of preceding group A
streptococcal infection + →
Either 2 of the major manifestations or
One major & 2 minor manifestations.
TREATMENT
❖ Bed rest

❖ Antibiotic Therapy:
10 days of orally administered penicillin or
erythromycin or a single intramuscular
injection of benzathine penicillin to eradicate
GABHS from the upper respiratory tract
Afterwards, the patient should be started on
long-term antibiotic prophylaxis
Anti-inflammatory Therapy:
Anti-inflammatory agents (salicylates,
corticosteroids).
PREVENTION